title: Transport enzymes and renal tubular acidosis.
abstract: By analogy to the findings with other transport disorders such as Bartter's or Liddle's syndrome, it might be expected that the various forms of renal tubular acidosis (RTA) could result from defects in H-ATPase or H-K-ATPase. However, the available data do not yet support such a simple explanation. With regard to distal RTA, inhibition of H-K-ATPase with inhibitors such as vanadate blocks the increase in enzyme activity observed with potassium depletion, but does not produce distal RTA. H-K-ATPase does not increase with metabolic acidosis, and inhibition of its activity does not decrease ammonium or total acid excretion unless K depletion is also present. Maleic acid administration produces proximal RTA along with other proximal tubular dysfunction in experimental animals. However, it acts by reducing Na,K-ATPase activity rather than by affecting specific H+ ion transporters. This is pertinent to the findings that Na,K-ATPase activity is reduced in obstructive uropathy. Although the acidification defect in this disorder has been ascribed to a defect in H-ATPase, Na-K-ATPase function is also impaired. Thus, the role of isolated defects in H+ transporters in the development of clinical acidification disorders remains to be elucidated.
authors.affiliation: Department of Medicine, Northwestern University Medical School and VA Chicago Healthcare System, IL, USA.
authors.forename: S K
authors.initials: SK
authors.lastname: Mujais
mesh_terms: D000141:Acidosis, Renal Tubular / Q000201:enzymology* / Q000209:etiology; D000818:Animals; D001692:Biological Transport; D002462:Cell Membrane / Q000201:enzymology; D017506:H(+)-K(+)-Exchanging ATPase / Q000378:metabolism*; D006801:Humans; D054328:Proton Pump Inhibitors; D006180:Proton-Translocating ATPases / Q000378:metabolism*; D051381:Rats; D000254:Sodium-Potassium-Exchanging ATPase / Q000378:metabolism*
chemical_list: D054328:Proton Pump Inhibitors; D017506:H(+)-K(+)-Exchanging ATPase; D006180:Proton-Translocating ATPases; D000254:Sodium-Potassium-Exchanging ATPase